Initially by Tim Slesnick 6/04


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Initially by Tim Slesnick 6/04

  1. 1. Initially by Tim Slesnick 6/04 Most recently revised 2/08
  2. 2. Goals Review both fetal and neonatal cardiac physiology Understand what murmurs are, how they occur, and how to describe them Discuss several types of congenital heart disease and how to distinguish them Review common genetic syndromes and their associated heart defects
  3. 3. Differences in the Fetus Foramen Ovale Ductus Arteriosus Right heart is the dominant ventricle – pumps 2/3 of cardiac output Relative RVH in utero
  4. 4. Differences in the Fetus
  5. 5. Differences in the Fetus
  6. 6. Changes after Birth Pulmonary Vascular Resistance begins to fall immediately Foramen Ovale closes – within the first hour Ductus Arteriosus closes – up to 48 hours can be normal Left heart now the dominant side Process continues for up to 6-8 weeks
  7. 7. Cardiac Evaluation History Exam: Inspection Palpation Auscultation
  8. 8. History Prematurity Maternal pregnancy complications (DM, PIH, infections, teratogen exposure) Abnormal ultrasounds Family history of congenital heart disease
  9. 9. History Infants symptoms: Tachypnea Diaphoresis Fatigue Cyanosis Especially if any symptoms with feeding (exercise for babies)
  10. 10. Physical exam – inspection and palpation Inspection Chest symmetric, normal shape Other systems (dysmorphic, edema, cyanosis, clubbing) Palpation PMI Thrills (palpable murmurs) Pulses (brachial and femoral)
  11. 11. Cardiac Exam – Auscultation Rate and rhythm Heart sounds Extra sounds Murmurs
  12. 12. What is a heart murmur? Results from turbulent blood flow, typically from the pressure difference between adjacent cardiac structures Can be normal (physiologic, benign, flow, transitional, etc) vs abnormal (pathologic) Most (80%) children will have soft murmurs in the perinatal period
  13. 13. Murmurs Location Radiation Timing (systolic, diastolic, continuous) Intensity (1-6 systolic, 1-4 diastolic) Pitch (high frequency [diaphragm better] vs low frequency [bell better]) Quality
  14. 14. Benign Murmurs Pulmonary flow (LUSB, soft) Peripheral pulmonary branch stenosis (axillae, back) Neonatal Still’s murmur (LLSB, “vibratory” or “musical”) Venous hum (continuous, under either clavicle but R more often than L)
  15. 15. Murmur Age Timing Location Character Pulmonary Flow Newborns early systole LUSB Medium to low pitch PPBS Newborns Mid-systole R or L mid sternal, radiates to back and axillae Med-high frequency Still’s Newborns, >50% 2 -7yo Early-mid systole, short murmur LUSB Musical, vibratory, buzzing Venous Hum > 50% young children Continuous, louder in diastole Infra- clavicular Blowing, low frequency
  16. 16. Murmur Increases Decreases Cause Confused with Pulmonary Flow ↑ CO ↓CO With valsalva Vibration at RVOT Pulmonary stenosis, ASD PPBS ↑ CO ↓CO Relative PS at 90º bifurcation of PA PS, ASD, LPA or RPA stenosis, coarctation Still’s ↑ CO (lying down, fever) Sitting up ?vibration at LVOT VSD, AS, LVOT obstruction, HCM Venous Hum Standing Turning head toward side listening to Turbulent flow in venous system Pulmonary AVM, PDA, breath sounds
  17. 17. RED FLAGS Diastolic murmurs (only venous hum is OK) Continuous murmurs (PDA should be gone by 48 hours) Loud murmurs + thrills SYMPTOMS, especially cyanosis
  18. 18. Pathologic murmurs Caused by abnormal anatomy or communications and the turbulent blood flow through them Typically from problems with valves (pulmonic stenosis, aortic stenosis), narrowings (coarctation) or holes where they shouldn’t be (VSD, ASD, PDA)
  19. 19. Acyanotic Heart Disease VSD May not hear at birth until PVR drops Typically holosystolic (engulfs S1 and S2) Typically loudest LLSB May have a thrill Louder murmurs are typically smaller holes (greater pressure difference)
  20. 20. Acyanotic Heart Disease Patent Ductus Arteriosus Continuous, “machine like” murmur Best under L clavicle Should disappear by 48 hours
  21. 21. Acyanotic Heart Disease Coarctation of the aorta Often can’t appreciate until ductus arteriosus closes, then rapid detioration Systolic ejection murmur best LUSB and over back Decreased femoral pulses
  22. 22. Cyanotic Heart Disease Most infants with cyanotic heart disease are cyanotic at birth, so shouldn’t be in Level II Check mucous membranes, nailbeds, etc (all infants can get perioral vascular congestion which isn’t real cyanosis) Caused by shunting of blood from the right to the left (deoxygenated blood) The 5 “Terrible T’s”
  23. 23. Truncus Arteriosus Only one vessel coming off the ventricles
  24. 24. Transposition of the Great Arteries Aorta off the RV, Pulmonary artery off the LV Must have mixing (ASD, VSD, PDA) or incompatible with life “Egg on a string” x-ray Often no murmur
  25. 25. Tricuspid Atresia (and Ebstein’s) Tricuspid valve is closed (atresia) or displaced and dysfunctional (Ebstein’s) HUGE heart on x-ray (mainly right atrium)
  26. 26. Tetralogy of Fallot VSD Overriding aorta Right ventricular hypertrophy Pulmonary stenosis “Boot shaped heart” on x- ray Murmur is from pulmonic stenosis, not from VSD
  27. 27. Total Anomalous Pulmonary Venous Return (TAPVR) Pulmonary veins come back somewhere besides the left atrium If obstructed, is the only pediatric cardiac surgical EMERGENCY CXR is “snowman in a snowstorm”
  28. 28. “Terrible” hypoplastic left heart syndrome Spectrum of disease, extreme form has almost no left ventricle, mitral atresia, aortic atresia, coarctation of the aorta As PDA closes, no blood to body – incompatible with life Often very non-specific physical exam, CXR
  29. 29. SVT
  30. 30. Vtach
  31. 31. Genetic syndromes associated with CHD Trisomy 13 PDA, septal defects, pulmonic and aortic stenosis Trisomy 18 VSD, polyvalvular disease, coronary abnormalities Trisomy 21 – 45% have heart defect AV canal, VSD, PDA, anomalous subclavian artery All need echo
  32. 32. More Syndromes Turner (XO) 30% bicuspid aortic valve; 10% coarctation Noonan pulmonary valve stenosis, ASD Hypertrophic cardiomyopathy in 20% DiGeorge/ VCF/ 22q11 Interrupted aortic arch, right aortic arch truncus arteriosus, tetrology of Fallot, pulmonary atresia with VSD
  33. 33. And more syndromes! Marfan: dilatation of ascending aorta/ aortic sinus, aortic and mitral insufficiency VACTERL: VSD in majority of cases Williams: supravalvular aortic stenosis, pulmonary artery stenosis
  34. 34. More syndromes again… Ellis-van Creveld: ASD or single Fetal Alcohol Syndrome: VSD Holt-Oram: atrial and ventricular septal defects, arrhythmias
  35. 35. Last page of syndromes! Pompe disease: (glycogen storage) cardiomyopathy MPS:  storage of MPS in arteries, valves w/ insufficiency and stenosis Hyperlipoproteinemia:  premature atherosclerosis Freidrich ataxia:  cardiomyopathy Muscular dystrophy: myocardial degeneration and fibrosis
  36. 36. Key Points Neonatal period, and particularly the first few days, are a time of great change Most murmurs are benign, but if its loud, harsh, diastolic, or the infant has symptoms, be concerned 1-2-3-4-5 cyanotic heart diseases Genetic syndromes have commonly associated heart defects
  37. 37. Any Questions ?